Saethre-Chotzen Syndrome: A Report of 7 Patients and Review of the Literature.

INTRODUCTION: Saethre-Chotzen syndrome is a genetic condition characterized by craniofacial and limb anomalies, with craniosynostosis (mainly coronal) being the most frequent craniofacial finding. Cranial and facial deformities can be extremely variable requiring individualization of treatment strategies. We present our case series to highlight clinical findings, treatment philosophy, and challenges facing Saethre-Chotzen patients. METHODS: A retrospective review was performed on records of patients given a diagnosis of Saethre-Chotzen syndrome at the University of California Los Angeles (UCLA) Craniofacial Clinic (n = 7) between 1980 and 2010. Patients with complete records were included in this study, and review of demographic data, clinical findings, surgical interventions and postoperative follow-up, and stability were performed. RESULTS: Seven patients (1 male and 6 female) were included in this study. The average age at which the patients were first seen was 6.5 years. Suture involvement was bicoronal (n = 6) and unicoronal (n = 1). There was 1 patient having superimposed metopic synostosis, and there was another patient having Kleeblattschädel deformity. Previous procedures performed for patients before establishing care at UCLA were strip craniectomy (n = 2) and fronto-orbital advancement (n = 2). All patients (n = 7) had fronto-orbital advancements at UCLA. Other skeletal operations included the following: redo forehead advancement and contouring (n = 3), monobloc advancement (n = 1), and LeFort III distraction (n = 1). Five patients reached skeletal maturity, and 2 patients received LeFort I advancement for class III malocclusion, one of which also required a bilateral sagittal split osteotomy of the mandible. CONCLUSION: Clinical presentation and severity of deformity in Saethre-Chotzen syndrome are variable. Our current report reviews our treatment strategies and illustrates the predominance of cranial and upper face deformities and frequent need for redo surgeries to address forehead asymmetry in this group of syndromic craniosynostosis patients.

The arrhinias: Proboscis lateralis literature review and surgical update.

Proboscis lateralis (PL) is a rare malformation, reported for the first time in 1861 by Forster in his monograph on congenital malformations of the human body. The abnormal side of the nose is represented by a tube-like rudimentary nasal structure, attached at any point along the embryonic fusion line between the anterior maxilla and the frontonasal processes. As clefts of the lip (and alveolus) are bilateral or unilateral, an arrhinia can be bilateral (total) or unilateral. In this case it is a 'hemi-arrhinia' (or heminasal agenesis. The arrhinias represent three groups of anomalies, each with different levels of clinical severity, some involving association with the labio-palatal cleft or agenesia of the premaxilla (1). In PL the nasal cavity on the affected side is replaced by a tubular appendage located off-center from the midline of the face, arising commonly from the medial aspect of the roof of the orbit (2). It is usually associated with heminasal aplasia or hypoplasia, microphthalmia, and - less commonly - with midline clefting. Associated brain and cranial vault anomalies are seen in 19% of these patients. PL is usually unilateral, with very few symmetrical/bilateral cases being reported (3). Morpho-aesthetic and psychological problems are frequent concerns for the patients and their families. In this study, the authors describe a clinical case and the chosen surgical technique, as well as reviewing the alternative techniques present in the literature.

Internal Distraction Resulted in Improved Patient-Reported Outcomes for Midface Hypoplasia.

BACKGROUND: Both internal and external distraction devices have been used successfully in correcting midface hypoplasia. Although the indication for surgery and the osteotomy techniques may be similar, deciding when to use internal versus external devices has not been studied. The authors studied patient-reported outcomes with FACE-Q and functional surveys for internal and external devices for midface distraction patients. METHODS: Patients who underwent distraction advancement after Le Fort I and Le Fort III were surveyed using the FACE-Q survey and a functional survey. Equal groups of internal and external device patients were compared (n = 64). Data recorded included: sex, age, follow-up, diagnosis, operating room time, expected blood loss, length of stay, distraction length, consolidation time, and complications. RESULTS: Internal and external device groups were similar with regards to patient diagnosis, operative time, expected blood loss, distraction length but consolidation times differed (internal = 3.6 versus external = 1.1 months). For FACE-Q appearance appraisal, there were similarities in domain and scale. For the functional survey (airway/breathing, ocular/vision, occlusion/eating, speech/articulation), there was also similar scoring. However, internal device patients had superior FACE-Q scores for Quality of Life: Social Function (80.9 versus 68.9), Early Life Impact (92.9 versus 62.4), Dental Anxiety (70.2 versus 48.3), Psychological Well-being (87.8 versus 68.6); and Decision Satisfaction (81.2 versus 56.9) and Outcome Satisfaction (91.0 versus 84.7). CONCLUSIONS: Internal and external midface distraction patients had similar patient-reported outcomes for appearance and functional improvement; however, internal device patients were more satisfied with their quality of life and their decision to undergo the procedure.

Necessity of latency period in craniofacial distraction: Investigations with in vitro microdistractor and clinical outcomes.

BACKGROUND: To determine the need for latency period in membranous bone distraction, we performed 1) in vitro comparison of preosteoblasts suspended in a 3D microdistraction model and 2) a clinical study comparing mandibular distraction cases with/without latency. METHODS: In the In Vitro study, Preosteoblasts polymerized in 3D-collagen gel were placed in a microdistractor and separated into three groups: 1) distraction with latency, 2) distraction without latency, and 3) static. After 2, 4, 6, and 8 days, cell proliferation, total protein levels, alkaline phosphatase activity, and osteogenic gene expression were assessed through RT-PCR. In the clinical study, patients underwent mandibular distraction in two groups: 1) latency and 2) no latency (n = 45). The rest of the distraction protocol was identical. Outcome was based on clinical examination, radiographs at six months, and 3D CT scans. RESULTS: In the In Vitro study, The distraction without latency group compared to the latency group had delays in: proliferation, total protein count, alkaline phosphatase activity, osteogenic gene expression in CBFA-1 (fourfold vs. eighteenfold), and in osteocalcin (twofold vs. sixfold). The distraction without latency group had higher apoptotic levels during the first four days compared to the latency group (68% vs. 14%). For the clinical study, similar perioperative complications (5% vs. 6%), X-ray mineralization (93% vs. 94%), bone volume, (8.6 vs. 9.1 cc) and bone density of central distraction zone (78% vs. 81%) were observed with or without latency. CONCLUSIONS: In vitro studies showed poorer results in cell survival, proliferation and osteogenic activity compared to distraction with latency; yet, clinically, there were no differences in distraction with latency versus without.

The Tessier number 3 cleft: a report of 10 cases and review of literature.

The Tessier number 3 cleft is one of the most intricate and destructive of all facial clefts, presenting surgeons with a difficult task for reconstruction. We present a series of 10 patients with this rare cleft all treated by a single surgeon over 30 years. All patients with Tessier number 3 clefts treated between 1978 and 2008 by the senior surgeon were reviewed. Demographic data and all associated clinical findings including cranial and extracranial anomalies were recorded. Methods used to reconstruct each patient were also noted. Seven males and three females were identified and age at initial treatment ranged from 12 months to 12 years. Mean follow-up was 6.3 years. Multiple craniofacial anomalies were appreciated including other rare facial clefts, hypertelorbitism, lacrimal obstruction, anophthalmia, choanal atresia, and hemifacial microsomia. Amniotic banding was the most prominent extracranial finding noted in these patients. Tessier number 3 clefts can be associated with multiple other craniofacial anomalies making reconstruction challenging. Soft tissue and bony reconstruction must be considered separately, and a variety of tools may be employed to accomplish each goal. As the presentation can be highly variable, an individualized treatment plan must be made to meet each patient's specific needs.

Maxillary hypoplasia in the cleft patient: contribution of orthodontic dental space closure to orthognathic surgery.

BACKGROUND: Cleft lip and palate surgery in the developing child is known to be associated with maxillary hypoplasia. However, the effects of nonsurgical manipulations on maxillary growth have not been well investigated. The authors present the contribution of orthodontic dental space closure with canine substitution to maxillary hypoplasia and the need for orthognathic surgery. METHODS: Cleft lip/palate and cleft palate patients older than 15 years of age were reviewed for dental anomalies, orthodontic canine substitution, and Le Fort I advancement. Skeletal relationships of the maxilla to the skull base (SNA), mandible (ANB), and facial height were determined on lateral cephalograms. Logistic regression analyses were performed to estimate odds ratios. RESULTS: Ninety-five patients were reviewed (mean age, 18.1 years). In 65 patients with congenitally missing teeth, 55 percent with patent dental spaces required Le Fort I advancement. In contrast, 89 percent who underwent canine substitution required Le Fort I advancement (p = 0.004). Canine substitution is associated with a statistically significant increase in maxillary retrognathia when compared with dental space preservation on lateral cephalograms (mean SNA, 75.2 and 79.0, respectively; p = 0.006). Adjusting for missing dentition, logistic regression analyses demonstrated that canine substitution is an independent predictor for orthognathic surgery (OR, 6.47) and maxillary retrusion defined by SNA < 78 (OR, 8.100). CONCLUSIONS: The coordination of orthodontia and surgery is essential to cleft care. The authors report a strong association between orthodontic cleft closure using canine substitution with maxillary hypoplasia and subsequent Le Fort I advancement, and suggest systematic criteria for management of cleft-related dental agenesis. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, III.

Parry-Romberg reconstruction: beneficial results despite poorer fat take.

OBJECTIVE: For the treatment of Parry-Romberg syndrome or progressive hemifacial atrophy, we studied the volume retention and skin changes after autologous fat grafts within diseased regions. SUMMARY BACKGROUND DATA: The long-term survival and volume retention of fat grafts used in soft tissue reconstruction of Parry-Romberg syndrome is still unknown, as are skin changes after fat grafting. METHODS: Sex, age, severity of deformity, number of procedures, operative times, and augmentation volumes were recorded. Preoperative/postoperative 3-dimensional computed tomographic scans were also reviewed. A digital 3-dimensional photogrammetry system was used to determine "final fat take" and symmetry. Romberg fat grafting volumes were compared to nonaffected, cosmetic fat-grafted patients. For skin changes, a spectrophotometer was used to quantify percent improvement in melanin index. Physician and patient satisfaction surveys (5-point scale) were elicited, including overall outcome and skin color/texture. RESULTS: The mean number of procedures correlated to the severity of deformity: mild, 1.8 procedures; moderate, 3.4; and severe, 5.2. With Romberg patients, fat grafting injected: per case, 48 mL; total, 188 mL; and final measured volume, 101 mL. Romberg patients had less "fat take" than nonaffected grafted patients (final take, 41% vs 81%). Skin color/texture showed 3-fold improvement after fat grafting procedures. The mean melanin index improvement seen in the diseased regions of Romberg patients after fat grafting was 42% (+3%). Skin color and texture improvement was also shown in patient surveys (preoperative = 2.4 + 0.06 to follow-up = 3.4 + 0.09) and physician (preoperatively = 2.1 + 0.1 to follow-up = 3.6 + 0.1). CONCLUSIONS: Despite poorer fat graft take within the disease region of Romberg patients, fat grafting resulted in long-term improvement in hypoplasia and skin hyperpigmentation.

Intraosseous venous malformations of the zygoma: clarification of misconceptions regarding diagnosis and management.

Primary intraosseous venous malformations affecting the zygoma are rare vascular lesions, with only 35 cases reported in the surgical literature. Despite the establishment of the binary classification system, which serves to distinguish vascular tumors from malformations, inappropriate use of the term "hemangioma" to describe a variety of distinct vascular anomalies remains widespread. The authors present 3 cases of zygomatic intraosseous venous malformations and summarize the clinical, radiographic, and immunohistochemical features of these lesions. In each case, an insidious clinical course, combined with the pathognomonic finding of radiating trabeculae on computed tomography, suggests the diagnosis of intraosseous venous malformation. Negative glucose transporter isoform 1 immunoreactivity and histopathological analysis were used to reinforce this diagnosis in 1 patient. Management was individualized in each case, based on symptom complex and aesthetic concern. Given that the therapeutic approach to vascular anomalies is dependent on accurate diagnosis, resolution of semantic matters will optimize the management of these lesions.

Radiation-induced craniofacial deformities: a new classification and management algorithm.

Little is written about the spectrum of late radiation-induced craniofacial abnormalities and the guidelines for treating these abnormalities. The clinical records of 13 patients (eight males and five females) who received childhood craniofacial radiation between birth and 11 years of age and who subsequently had reconstructive surgery were reviewed. Eleven patients had their irradiation at the age from 1 to 5 years. The other two patients received their treatment at a relatively older age (9 and 11 years). Their deformities ranged from isolated soft-tissue deficiency with no or minimal bony deficiency to cases having osseous deformities with or without soft-tissue deficiency but still the normal or near-normal craniofacial form can be obtained with surgical intervention and the outermost extreme of the deformity is the patients whose normal or near-normal craniofacial form and function cannot be regained even with much sophisticated surgeries. Our new classification is based on two factors: the tissue component of the deformity and the possibility of regaining a normal or near-normal craniofacial form and function with the planned surgical intervention. Based on this classification, a new treatment algorithm was created.

Fibrous dysplasia of the zygomaticomaxillary region: outcomes of surgical intervention.

BACKGROUND: Fibrous dysplasia is the most common craniofacial tumor, presenting in both monostotic and polyostotic forms with varying degrees of severity. No consensus exists regarding the surgical management of craniofacial fibrous dysplasia, particularly in the zygomaticomaxillary region. The present study compared long-term outcomes of limited reduction burring versus radical resection of zygomaticomaxillary fibrous dysplasia. METHODS: Patients with craniofacial fibrous dysplasia at the University of California, Los Angeles, Craniofacial Center from 1982 to 2008 were studied based on demographics, treatment, and follow-up data, including examinations, computed tomographic scans, photographs, physician Whitaker scoring, and patient surveys (n=97). Outcomes were compared for zygomaticomaxillary disease treated with radical resection with cranial bone graft reconstruction or limited reduction burring (n=58). RESULTS: Thirty-four percent of patients had monostotic disease, 66 percent had polyostotic disease, 3 percent had McCune-Albright syndrome, and 2.1 percent had malignant degeneration into osteosarcoma. Most patients had surgical treatment (84.5 percent). Of the patients that required optic nerve decompression for vision changes (11.4 percent), most (75 percent) had vision stabilization postoperatively. Differences were recorded in zygomaticomaxillary disease treated with radical resection (63.8 percent) versus reduction burring (36.2 percent) according to age (19.6 versus 14.2 years), complications (13.5 percent versus 4.8 percent), recurrence (66.7 percent versus 24.3 percent), and number of subsequent procedures (2.8 versus 4.0). There were similarities in Whitaker outcome score (1.3±0.3 versus 1.5±0.6) and patient satisfaction (2.7±0.4 versus 2.8±0.3). CONCLUSIONS: Although different approaches have been advocated to treat fibrous dysplasia, the authors' data support a more aggressive management for zygomaticomaxillary disease with radical resection and cranial bone graft reconstruction, especially for more involved disease. CLINICAL QUESTION OF EVIDENCE: Therapeutic, III.

Soft-tissue volumetric changes following monobloc distraction procedure: analysis using digital three-dimensional photogrammetry system (3dMD).

We have previously reported that monobloc advancement by distraction osteogenesis resulted in decreased morbidity and greater advancement with less relapse compared with acute monobloc advancement with bone grafting. In this study, we examine the three-dimensional (3D) volumetric soft-tissue changes in monobloc distraction.Patients with syndromic craniosynostosis who underwent monobloc distraction from 2002 to 2010 at University of California-Los Angeles Craniofacial Center were studied (n = 12). We recorded diagnosis, indications for the surgery, and volumetric changes for skeletal and soft-tissue midface structures (preoperative/postoperative [6 weeks]/follow-up [>1 year]). Computed tomography scans and a digital 3D photogrammetry system were used for image analysis.Patients ranged from 6 to 14 years of age (mean, 10.1 years) at the time of the operation (follow-up 2-11 years); mean distraction advancement was 19.4 mm (range, 14-25 mm). There was a mean increase in the 3D volumetric soft-tissue changes: 99.5 ± 4.0 cm(3) (P < 0.05) at 6 weeks and 94.9 ± 3.6 cm(3) (P < 0.05) at 1-year follow-up. When comparing soft-tissue changes at 6 weeks postoperative to 1-year follow-up, there were minimal relapse changes. The overall mean 3D skeletal change was 108.9 ± 4.2 cm. For every 1 cm of skeletal gain, there was 0.78 cm(3) of soft-tissue gain.Monobloc advancement by distraction osteogenesis using internal devices resulted in increased volumetric soft-tissue changes, which remained stable at 1 year. The positive linear correlation between soft-tissue increments and bony advancement can be incorporated during the planning of osteotomies to achieve optimum surgical outcomes with monobloc distraction.

Constructing the philtral column in the secondary cleft lip deformity: utilizing the palmaris longus graft.

BACKGROUND: The philtrum and the Cupid's bow are the most prominent features of the upper lip and are crucial for normal appearance. Unfortunately, projection of the philtral column is a common deficiency of the repaired cleft lip. Although a multitude of methods for constructing the philtral dimple and ridge have been described, no single procedure has achieved complete satisfactory results. The authors describe their technique for construction of the philtral column using palmaris longus tendon. METHODS: Between 1998 and 2009, a retrospective study was performed for identifying patients with depression of the philtral scar following cleft lip repair. Pre- and postoperative photographs were taken with at least 6 months follow-up. Postoperative patient satisfaction and occurrence of complications are reported. RESULTS: In all, 17 patients who developed depression of the philtral ridge following cleft lip repair underwent philtral reconstruction utilizing palmaris longus tendon grafts by the senior author (H.K.K.). At the time of surgery, average age for 11 females and 6 males was 18.5 years. The follow-up period ranged from 6 to 126 months, with an average of 42.3 months. In the early postoperative period, 2 complications in the form of graft exposure occurred. However, both cases were successfully managed with preservation of the graft. All patients were satisfied with their long-term results, and none required further surgery. CONCLUSIONS: The use of palmaris longus tendon graft is an effective, reproducible, and safe method by which to construct the philtral column in the secondary cleft lip deformity.

Craniofacial surgical strategies for the correction of pneumosinus dilatans frontalis.

INTRODUCTION: Pneumosinus dilatans is a rare condition and different techniques have been proposed for its management and correction. The abnormally expanded, aerated frontal sinus has been described in the literature as: frontal sinus hypertrophy, pneumosinus dilatans, pneumosinus frontalis, aerocele, pneumocele, sinus ectasia, hyperpneumatization and others. The precise aetiology and pathogenesis of the condition is unknown, although several basic hypotheses have been proposed MATERIAL AND METHODS: The authors report two cases of frontal bossing and supraorbital ridge deformity correction using craniofacial surgical principles. DISCUSSION: Functional and morphological results are discussed and compared with other open procedures. CONCLUSION: A variety of surgical procedures have been proposed for the correction of the pneumosinus dilatans frontalis. The craniofacial approach is advocated to reproduce the normal anatomy of the forehead in the upper part, the supraorbital rim and glabellar area.

Le Fort II osteotomy.

BACKGROUND: In comparison with the abundant literature on Le Fort I and III osteotomies, there is scant information on the Le Fort II osteotomy. Our goal in this study was to define the indications and techniques of the elective Le Fort II osteotomy. We reviewed our 30-year experience, which is the longest series of patients treated with Le Fort II osteotomies at a single institution. METHODS: A review of the records of 13 consecutive patients at our institution with a diagnosis of Le Fort II was conducted. Data analyzed included demographic information, indications, techniques, complications, and combined procedures. On the basis of surgical outcomes, a treatment algorithm was created. RESULTS: Thirteen patients had a diagnosis of Le Fort II at our institution. Five were excluded because of incomplete records or Le Fort II fracture repair. Three patients were male, and 5 were female. Their average age was 23 years, and the average follow-up was 60 months. Indications included nasomaxillary deviation related to unilateral coronal synostosis, hemifacial microsomia, Romberg disease, and noncleft nasomaxillary hypoplasia. The average estimated blood loss was 950 mL. Complications were persistent nasal deviation, temporary nasal obstruction, and a hematoma. Complementary procedures included bilateral sagittal split osteotomies, bone grafts, Le Fort I osteotomy, and repositioning of the zygoma. CONCLUSIONS: On the basis of this review, we have elucidated the indications and less invasive techniques of the elective Le Fort II osteotomy. No major complications were observed. A management algorithm for performing the Le Fort II osteotomy is presented.

Parry-Romberg reconstruction: optimal timing for hard and soft tissue procedures.

For the treatment of Parry-Romberg syndrome or progressive hemifacial atrophy, we studied 3 controversial issues: (1) optimal timing, (2) need for skeletal reconstruction, and (3) need for soft tissue (medial canthus/lacrimal duct) reconstruction. Patients with Parry-Romberg syndrome (>5 y follow-up) were divided into 2 groups: (1) younger than 14 years and (2) 14 years or older (n = 43). Sex, age, severity of deformity, number of procedures, operative times, and augmentation fat volumes were recorded. Physician and patient satisfaction surveys (5-point scale) were obtained, preoperative and postoperative three-dimensional computed tomographic scans were reviewed, and a digital three-dimensional photogrammetry system was used to determine volume retention. Our results indicate that the younger patient group required more procedures compared with the older patient group (4.3 versus 2.8); however, the younger group had higher patient/family satisfaction scores (3.8 versus 3.0). Skeletal and soft tissue reconstruction resulted in improved symmetry score (60% preoperatively to 93% final) and satisfaction scores (3.4 preoperatively to 3.8 final). Patients with Parry-Romberg syndrome required multiple corrective surgeries but showed improvements even when beginning before puberty. Soft and hard tissue reconstruction was beneficial.

Optimizing the timing and technique of Treacher Collins orbital malar reconstruction.

The optimal timing and treatment of Treacher Collins syndrome with regard to zygomatico-orbital osseous reconstruction has not been fully established. Osseous reconstruction performed at an early age may result in bone graft resorption; however, delays in surgical improvement may result in adverse psychosocial effects on the patient. To study the optimal age for reconstruction clinically, we examined 3 age groups based on timing of malar and eyelid reconstruction using a three-dimensional computed tomographic scan and three-dimensional photometric volume assessment. In addition, we collected outcome assessments from parents/patients using satisfaction surveys. Of 73 patients with Treacher Collins at the University of California Los Angeles Craniofacial Clinic, 45 had malar reconstruction, complete records, and were available for study. The patients were separated into 3 groups: (1) very young = 0 to 5 years, (2) mid-childhood = 6 to 12 years, and (3) adolescent/adult 13 years and older. As hypothesized, the 0 to 5 age group experienced the greatest percentage of complete bony resorption (77%) compared with the 6 to 12 age group (only 4%) and the 13 and older age group (0%). Interestingly, the 0 to 5 age group experienced the highest parent/patient satisfaction, possibly owing to better psychosocial experience. When dealing with treatment plans for patients with Treacher Collins, patients, parents, and physicians must balance the benefits of early surgical intervention, such as improved psychologic well-being, with the disadvantages of having to undergo a greater number of procedures, such as fat grafting after malar bone graft resorption.

Orthognathic surgery for obstructive sleep apnea: applying the principles to new horizons in craniofacial surgery.

This article is dedicated to the senior author Dr. Henry K. Kawamoto, Jr, who pioneered the use of orthognathic surgery to treat severe obstructive sleep apnea in 1981. Since that time, his techniques for maxillomandibular advancement have been revised and expanded for improved surgical success. Obstructive sleep apnea is a growing public health concern because it can cause hypertension, cardiac arrhythmias, heart attack, stroke, and, in rare circumstances, sudden death if untreated. When less invasive options fail such as weight loss or dental devices for mandibular repositioning, maxillomandibular advancement is a valuable treatment option for severe obstructive sleep apnea.

18-gauge needle cap as adjunct to prevent kinking of endotracheal tube.

A self-retaining Dingman mouth retractor is widely used to keep the mouth open during cleft palate and intraoral surgery. The airway is at risk of being crushed or occluded as the gag (tongue plate) of the Dingman mouth retractor is being pushed against the endotracheal tube.Kinking of the endotracheal tube between the teeth and Dingman mouth retractor has been reported even with the oral Ring-Adair-Elwyn or flexometallic or armored endotracheal tubes. To minimize kinking of the endotracheal tube and its consequent complications, we routinely insert an 18-gauge needle cap at the potential space between the teeth and the tongue plate (gag) of the Dingman mouth retractor, which is situated lateral to the endotracheal tube. In our experience of approximately 5000 intraoral cases using a Dingman mouth retractor and 18-gauge needle cap, we have not had any tooth avulsion or aspiration of the 18-gauge foreign body while maintaining a consistent and secured airway during cleft palate and intraoral surgery.

Asymmetric class III malocclusion: association with cranial base deformation and occult torticollis.

The etiology of Angle class III malocclusion with facial asymmetry has not been fully elucidated. To investigate the etiology, patients with asymmetric prognathism (n = 30) from a single institution were assessed for previously undiagnosed torticollis and cranial base asymmetry. Presence of torticollis was determined by measuring restricted head movement when turning the head against a wall and cranial base tilt with upward gaze. Cranial base asymmetry was evaluated by preoperative three-dimensional computed tomography scans. Thirty-one percent of patients with prognathism presented with concurrent facial asymmetry. In patients with asymmetric prognathism, cranial base tilt was present on upward gaze in all patients; mean angle between head and wall was 31 degrees greater than that in control patients, and a 22% to 36% difference in the angle was present when comparing one side with the other. Based on these findings, all patients with asymmetric prognathism were found to be affected by torticollis. By computed tomography scan, 85% of these torticollis patients showed slight anteromedial displacement of the glenoid fossa ipsilateral to torticollis, and 73% demonstrated temporal fossa shift of 4 mm or greater. The current study demonstrates a strong association between asymmetric class III malocclusion, torticollis, and cranial base asymmetry. We conclude that undiagnosed torticollis is a likely etiology for otherwise idiopathic cranial base asymmetry and that cranial base asymmetry in turn causes facial asymmetry and malocclusion. This study highlights the importance of evaluating cranial base asymmetry and torticollis in patients with class III malocclusion to allow for earlier treatment and improved outcomes.